Ávila Pablo, Chaix Marie-A, Mondésert Blandine, Khairy Paul
Department of Cardiology, Instituto de Investigación Sanitaria, Hospital Gregorio Marañón, Universidad Complutense, Madrid, Spain.
Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, 5000 Belanger Street East, Montreal H1T 1C8, Canada.
Card Electrophysiol Clin. 2017 Jun;9(2):225-234. doi: 10.1016/j.ccep.2017.02.003. Epub 2017 Mar 18.
Sudden death of presumed arrhythmic etiology is a leading cause of mortality in adults with congenital heart disease. Anticipated benefits of the implantable cardioverter-defibrillator (ICD) must be weighed against high complication rates. Without robust evidence from randomized trials, caregivers face difficult decisions in selecting appropriate candidates. Although secondary prevention indications are often clear-cut, risk stratification for primary prevention ICDs is more challenging. Factors associated with sudden death in patients with tetralogy of Fallot are reasonably consistent across studies. In contrast, identification of high-risk patients with systemic right ventricles or univentricular hearts remains controversial.
推测为心律失常病因的猝死是先天性心脏病成人患者的主要死亡原因。必须在植入式心脏复律除颤器（ICD）的预期益处与高并发症发生率之间进行权衡。由于缺乏来自随机试验的有力证据，护理人员在选择合适的候选人时面临艰难的决策。虽然二级预防指征通常很明确，但一级预防ICD的风险分层更具挑战性。法洛四联症患者猝死相关因素在各项研究中较为一致。相比之下，识别系统性右心室或单心室心脏的高危患者仍存在争议。
